Predictive significance of pretreatment 18F-FDG PET volumetric parameters on survival outcomes in pediatric patients with locally advanced undifferentiated nasopharyngeal carcinoma.

BACKGROUND: Nasopharyngeal carcinoma (NPC) is a rare pediatric cancer. Most children are first diagnosed with advanced locoregional disease. Identification of patients at higher risk of treatment failure is crucial as they may benefit from more aggressive initial treatment approaches. 18Fluorine-labeled fluoro-2-deoxyglucose positron emission tomography (18F-FDG PET) has shown promise as a prognostic tool for predicting outcomes. METHODS: Retrospective study of pediatric patients with locally advanced undifferentiated NPC who underwent 18F-FDG PET/CT prior to intial treatment. Predictive significance of metabolic PET parameters on survival outcomes were estimated. RESULTS: Thirty-two children were included, age range was 7.1-18 years at the time of diagnosis. The median follow-up duration was 46.1 months. Three patients (9.4%) were classified as AJCC stage IIb, 13 patients (40.6%) as stage IIIa, eight patients (25%) as stage IIIb, and eight patients (25%) as stage IVa. Our findings revealed that high whole-body metabolic tumor volume at the threshold of hepatic reference SUVmean (WB-MTV-HR) (>135 mL) was associated with significantly lower event-free survival (EFS) compared to the low WB-MTV-HR group (≤135 mL) (3-year EFS: 50% ± 18% vs. 82% ± 8%; p = .015). Additionally, the 3-year overall survival (OS) rates differed significantly between the high whole-body metabolic tumor volume at the threshold of an SUV of 2.5 isocontour (WB-MTV-2.5) group (MTV >74 mL) and the low WB-MTV-2.5 group (MTV ≤74 mL) (63% ± 18% vs. 100%; p = .021). CONCLUSION: Our study suggests that WB-MTV parameters could serve as significant prognostic factors for disease progression in pediatric patients with locally advanced undifferentiated NPC. However, further prospective studies with larger sample sizes are needed to validate these findings.

Prognostic value of detection of CD99+ , CD45- cells in peripheral blood by flow cytometry in children with Ewing sarcoma.

BACKGROUND: Early detection of metastasis and recurrence of Ewing sarcoma (ES) is important for early management. This work aimed to detect CD99+ , CD45- cells in peripheral blood by flow cytometry (FC) before and during chemotherapy and evaluate their prognostic significance. PROCEDURE: This prospective cohort study was carried out on 60 children newly diagnosed with ES at Children Cancer Hospital-Egypt 57357 and 40 healthy children control group. Detection of CD99+ , CD45- cells in peripheral blood was accomplished by FC at baseline before treatment and after five cycles of chemotherapy. Samples were classified as positive if they had more than the upper limit of cells observed in the control cases. Correlation between FC results and relapse and overall survival (OS) after one year was performed. RESULTS: Median percentage of CD99+ , CD45- cells was significantly increased in patients compared with controls (0.002% vs 0%, respectively, P < 0.001). Post-cycle 5 CD99+ , CD45- cells were increased in 12 patients, of them 11 patients' disease had either relapsed or progressed. Post-cycle 5 CD99+ ; CD45- cells had a 73.3% sensitivity and 97.8% specificity for predicting relapse or progression, whereas baseline only had 6.7% sensitivity and 77.8% specificity. The hazard ratio for mortality in the post-cycle 5 positive group was 18.4 [95% confidence interval (1.86 to 181.46)] times that of the negative group. One year OS was 91.67%. CONCLUSION: Post-cycle 5 CD99+ , CD45- cells in peripheral blood by FC is a strong predictor for relapse, progression, and mortality whereas baseline is a poor predictor in newly diagnosed patients with ES.

Prognostic significance of survivin expression in pediatric ewing sarcoma.

Survivin is an inhibitor of apoptosis protein that inhibits caspases and blocks cell death. It is undetectable in most normal adult tissues. High survivin expression has been detected in various tumors and has been correlated with therapy resistance and poor outcome. We conducted this study to examine survivin expression in pediatric Ewing sarcoma (ES) and evaluate its role in predicting clinical outcome. Formalin-fixed paraffin-embedded tumor tissues from 108 pediatric ES patients were examined by immunostaining with survivin rabbit monoclonal antibodies. Survivin was detected in tumor tissues of 72 (66.7%) patients. High expression (≥50%) was detected in 18 (16.7%) patients. High survivin expression was shown to be a significant univariate parameter for poorer overall (OS) and event free survival (EFS) with p value 0.033, and 0.037 respectively. It was confirmed as an independent factor in multivariate analysis for OS (p: 0.041; HR: 1.97 with 95% CI of 1.03-3.79) and EFS (p: 0.049; HR: 1.86 with 95% CI of 1.00-3.46). These results suggest that high survivin expression identifies a group of patients with poor prognosis and this may help to refine risk adapted treatment; however, this needs to be confirmed in prospective studies.

Temporal trends in childhood cancer survival in Egypt, 2007 to 2017: A large retrospective study of 14 808 children with cancer from the Children's Cancer Hospital Egypt.

Childhood cancer is a priority in Egypt due to large numbers of children with cancer, suboptimal care and insufficient resources. It is difficult to evaluate progress in survival because of paucity of data in National Cancer Registry. In this study, we studied survival rates and trends in survival of the largest available cohort of children with cancer (n = 15 779, aged 0-18 years) from Egypt between 2007 and 2017, treated at Children's Cancer Hospital Egypt-(CCHE), representing 40% to 50% of all childhood cancers across Egypt. We estimated 5-year overall survival (OS) for 14 808 eligible patients using Kaplan-Meier method, and determined survival trends using Cox regression by single year of diagnosis and by diagnosis periods. We compared age-standardized rates to international benchmarks in England and the United States, identified cancers with inferior survival and provided recommendations for improvement. Five-year OS was 72.1% (95% CI 71.3-72.9) for all cancers combined, and survival trends increased significantly by single year of diagnosis (P < .001) and by calendar periods from 69.6% to 74.2% (P < .0001) between 2007-2012 and 2013-2017. Survival trends improved significantly for leukemias, lymphomas, CNS tumors, neuroblastoma, hepatoblastoma and Ewing Sarcoma. Survival was significantly lower by 9% and 11.2% (P < .001) than England and the United States, respectively. Significantly inferior survival was observed for the majority of cancers. Although survival trends are improving for childhood cancers in Egypt/CCHE, survival is still inferior in high-income countries. We provide evidence-based recommendations to improve survival in Egypt by reflecting on current obstacles in care, with further implications on practice and policy.

Pediatric Nasopharyngeal Carcinoma: A Rare Tumor in a Developing Country-What Do We Learn?

BACKGROUND/AIM: Nasopharyngeal carcinoma (NPC) is rare in children, accounting for 1% of pediatric malignancies. The 5-fluorouracil Cisplatin regimen could be considered as a standard of care induction chemotherapy followed by concomitant chemoradiotherapy. This study aimed at detecting the survival outcome in correlation with different prognostic factors together with the toxicity of different treatment modalities. PATIENTS AND METHODS: This was a retrospective study carried out from 2007 to 2016 that included all NPC patients below 18 years treated at the National Cancer Institute, Egypt. RESULTS: A total of 21 patients were included with a median follow-up period of 33.9 months. The median age was 14.8 years (range: 9 to 18). All patients were treated by neoadjuvant chemotherapy with cisplatin and 5- fluorouracil, followed by concurrent radiotherapy (median dose: 61.2 Gy) and cisplatin as a radiosensitizer. After induction chemotherapy, the response rate was 53%. After completion of treatment, 67% had a complete response; partial response was seen in 14%; and progressive disease was seen in 19%. By the end of the study, 7 (33.3%) patients had progression/relapse; 4 of them died from disease. The 3-year overall survival and event-free survival were 85.7% and 66.7%, respectively. CONCLUSIONS: Neoadjuvant chemotherapy followed by concurrent chemoradiotherapy was an effective strategy in the treatment of pediatric NPC with good overall survival and event-free survival. High systemic failure (33.3%) remains another challenge to solve. More efforts should be made to improve survival by developing more efficient systemic treatment modalities, especially for progressive/relapsed disease. Multicenter studies on a larger number of patients are needed to identify different prognostic factors and standardize treatment strategies.

Management and outcome of pediatric Wilms tumor with malignant inferior Vena cava thrombus: largest cohort of single-center experience.

BACKGROUND: Wilms tumor (WT) with an inferior Vena cava (IVC) malignant thrombus comprises 4-10% of all WT cases. METHODS: This retrospective analysis included 51 pediatric patients presenting at Children Cancer Hospital Egypt-57357 from July 2007 to December 2016 with the diagnosis of WT with malignant IVC thrombus. RESULTS: Median age at presentation = 4.4 years and 28 cases (55%) were females. Twenty-five patients (49%) were metastatic and 4 patients (7.8%) had bilateral disease. Forty-seven cases (92.2%) had favorable histology with no evidence of anaplasia. Level of thrombus extension at presentation was classified as infra-hepatic, retro-hepatic, supra-hepatic and intra-cardiac in 33, 9, 6 and 3 patients, respectively. Fifty patients started neoadjuvant chemotherapy (CTH) with 16 patients showing complete resolution of thrombus after 6 weeks of CTH. None of the patients developed thrombus progression after neoadjuvant CTH; one patient had stationary intra-cardiac thrombus, while remaining patients showed partial regression of their thrombus and had nephrectomy with en-bloc thrombectomy. The mean cranio-caudal dimension of IVC thrombi at initial presentation was 6.5 cm, and 3.6 cm post 6th week of CTH. The 5-year OS and EFS were 75.9% and 71.1%, respectively. There was no significant correlation of initial levels of thrombus extension with survival. CONCLUSION: Neoadjuvant chemotherapy followed by radical nephrectomy with en-bloc thrombectomy and radiotherapy seems a successful approach for management of patients with WT and IVC tumor thrombus. Measurement of the cranio-caudal dimension of thrombus and its response to treatment should be considered in the surgical planning.

The outcome of childhood adrenocortical carcinoma in Egypt: A model from developing countries.

Adrenocortical carcinoma (ACC) is a rare, aggressive endocrine neoplasm. Complete surgical resection is the single most important treatment. Most available information has been learned from experience with its more frequent adult counterpart. In this study, we assessed the features and survival outcome of patients with ACC at Children's Cancer Hospital Egypt (CCHE). Patients diagnosed at CCHE between July 2007 and November 2016 were followed up on until November 2018. Patients with stages I and II were operated upon, while stages III and IV had received combinations of doxorubicin, etoposide, platinol, and mitotane (DEPM) beside the attempt to conduct surgery when feasible. Data belonging to 18 patients (7 men and 11 women) were analyzed; median age at diagnosis was 48.5 months. Sixteen patients had presented with secreting tumors. Six patients were diagnosed with stage I disease; four with stage II; three with stage III; and five with stage IV carcinoma. By the end of this study, 10 patients have survived; five-year overall survival of 66.3%. Surviving patients were all of stage I or II diseases and were all in remission. Seven patients who did not survive died due to tumor progression, while one patient died after chemotherapy. The prognosis of ACC is essentially dependent on a successful complete resection of the tumor and thus on the initial tumor stage. The mitotane and DEP protocols may help control tumor growth in the advanced stages for only short periods. Key pointsInitial stage and resectability are the main indicators of outcomes in adrenocortical carcinoma.Chemotherapeutic agents used in developed countries did not achieve the same outcomes.Further molecular-pharmacology differentiation is needed for various ethnic populations.

Risk of second malignancies among survivors of pediatric thyroid cancer.

BACKGROUND: Thyroid carcinoma is a very rare tumor in the pediatric age group, accounting for only 1.5-3% of childhood carcinomas in the United States and Europe. We aimed to identify the risk of a second malignancy among pediatric thyroid cancer survivors. METHODS: The cohort analysis consisted of pediatric cancer patients aged less than 20 years, diagnosed with a primary thyroid cancer, identified by site code ICD-0-3: C739, and reported to the SEER 9 database between 1973 and 2013. They were followed up until December 31, 2013; the end of the study period, or up to death if earlier. RESULTS: Out of 1769 patients diagnosed primarily with thyroid carcinoma, 42 patients had a total of 45 incidences of subsequent malignancies. The mean age of patients at the initial diagnosis of thyroid cancer was 16 years. Females (90.5%) had a significantly higher incidence of second malignancies (SM) than males (9.5%). The overall Standardized Incidence Ratio (SIR) of SM in the study patients was higher than expected (SIR = 1.48). Some specific sites showed significantly higher incidences: the salivary glands (SIR = 33.95), the gum and other parts of the mouth [excluding the lips, tongue, salivary glands and floor of the mouth] (SIR = 24.53)*** and the kidneys (SIR = 5.72). The overall risk of SM in patients who had received radioactive iodine was higher than expected (SIR = 4.41). The cumulative incidence of SM after treatment of thyroid cancer in children increases steadily over 40 years (11.92%). CONCLUSIONS: Race, gender, histological subtypes, and radioactive iodine are potentially significant prognostic factors for the development of SM among pediatric thyroid cancer survivors. Identification of underlying mechanisms that raise the risk of SM is important for both treatment and follow-up strategies.

Nonfunctioning Adrenocortical Carcinoma in Pediatric Acute Lymphoblastic Leukemia: A Case Report of a Rare Multiple Primaries Combination.

Childhood adrenocortical carcinoma (ACC) is a rare tumor and its association with acute lymphoblastic leukemia (ALL) is even rarer. One such case is discussed in this case report. A 3-year-old patient was concomitantly diagnosed with ALL and an initially nonmetastatic ACC. Management started by following the Total XV protocol without a window phase. Left adrenalectomy was conducted after the consolidation phase. Recurrence of a mass at the tumor bed was discovered at week 33 of the continuation phase. Reexcision was conducted, followed by the administration of an ACC protocol including cisplatin, etoposide, and doxirubicin. Mitotane was added when a pulmonary metastasis was discovered and then stopped after the patient suffered from an arachnoid cyst and speech difficulties. The ALL protocol was resumed from week 34 of the continuation phase. Progression of pulmonary nodules was noted after week 45. A pulmonary metastectomy was performed. The ALL protocol was resumed up to week 51 with a good response as proven by assessment of minimal residual disease. A further recurrence was diagnosed at the abdominal tumor bed with a paravertebral mass and a pulmonary nodule. The patient was assigned to palliative treatment and died after a 32-month survival. Such rare associations need more extensive discussions of the best possible management in scientific literature.

Phthisis bulbi: clinical and pathologic findings in retinoblastoma.

PURPOSE: Phthisis bulbi represents an ocular end-stage disease characterized by shrinkage and disorganization of the eye. We aim at identifying the pathologic changes of phthisis bulbi associated with retinoblastoma. DESIGN: Retrospective observational case series study. METHODS: 16 enucleated eyes were enrolled retrospectively between 2007 and 2012. Pathologic gross and microscopic findings were assessed. RESULTS: Cases showed grossly shrunken eyes with a mean volume of 4.3 cc. Sclera was markedly thickened in the majority of cases with mean of 2272.8 µ. Choroid showed an average thickness of 1029 µ. Necrosis, dystrophic calcification, ossification, gliosis, residual viable tumor was identified in many cases. Pathologic high risk factors were seen in three specimens. CONCLUSIONS: We conclude that retinoblastoma must be considered in the differential diagnosis of phthisis bulbi in the pediatric patients and active tumor was present in half of the patients.

Clear cell sarcoma of the kidney: patients' characteristics and improved outcome in developing countries.

BACKGROUND: Clear cell sarcoma of the kidney (CCSK) is a rare and aggressive tumor accounting for 5% of pediatric renal tumors with an incidence of 20 patients per year in the USA. It is bone metastasizing with poor prognosis. Our aim was to show characteristics of patients in relation to improved outcome in one of the developing countries. PROCEDURE: We included all patients diagnosed as CCSK in the period between July 2007 and March 2012 at Children's Cancer Hospital, Egypt. Patients' demographics, clinical presentation, pathology, and management were reviewed. Follow up was continued until April 2013. RESULTS: Twenty-five patients were identified in the defined time interval, accounting for 7% all renal tumors diagnosed at the hospital. Mean age was 36 months. Abdominal swelling and hematuria were the most common presentations. Stages I, II, III, IV, and V represented 9 (36%), 3 (12%), 8 (32%), 3 (12%), and 2 (8%), respectively. Twenty-four patients had radical nephrectomy either upfront or after neo-adjuvant chemotherapy. Surgery was followed by adjuvant chemotherapy. Abdominal radiotherapy was given for local stages II and III. Twenty-two patients reached complete remission, while one patient had stationary disease and two patients died due to progression and relapse. Overall survival was 88.5% and event-free survival was 87.8% at 45 months. CONCLUSION: Although previous studies indicate poor prognosis of CCSK, our experience shows that those patients can be treated using extensive chemotherapy combined with proper local control.

Impact of physical activity on postural stability and coordination in children with posterior fossa tumor: randomized control phase III trial.

BACKGROUND: Posterior fossa tumor is a type of brain tumor that is located at the borders of both the brain stem and cerebellum. The cerebellum is the brain region in charge of balance and coordination. Pediatric patients diagnosed with posterior fossa tumor have been reported to fall frequently. OBJECTIVES: The aim of this study is to investigate the effectiveness of balance and coordination training in these children. METHODS: This randomized control clinical trial (ClinicalTrials.gov Identifier: NCT04528316) was carried out between September 2020 and April 2021 at Children's Cancer Hospital-57357. The inclusion criteria were patients with posterior fossa tumor in maintenance phase and, age between 5 and 12 years. The exclusion criteria were patients who had a genetic disorder or suffer from mental retardation, a chronic lung disease, severe cardiomyopathy, or a neuromuscular disease that does not relate to tumor. The study participants were randomly assigned into three groups: Group I/Control group: they received Pilates core stability exercises program, Group II/Postural stability group: they received the same program plus HUMAC balance program, and Group III/Coordination group: they received the same program plus coordination exercises of BOT-2. The semi-parametric proportional odds model was used to compare follow-up scores of the Postural stability group vs Control, and Coordination group vs Control, while adjusting for baseline values. All tests were two sided, with alpha set to 0.05. RESULTS: Sixty children including 38 boys and 22 girls were enrolled in this study. In all three groups, postural stability and coordination improved significantly in terms of modified clinical test of sensory integration of balance, center of pressure, limits of stability, bilateral coordination, and upper-limb coordination. CONCLUSION: The current study supports the value of adding postural stability and coordination training to the physiotherapy plan for children with posterior fossa tumor. TRIAL REGISTRATION NUMBER AND DATE OF REGISTRATION: ClinicalTrials.gov Identifier: NCT04528316 on August 27, 2020.

Parotid gland masses: outcomes in the pediatric age group.

BACKGROUND: Childhood parotid neoplasms appear to have different characteristics from adults. This point, in addition to the rarity of these tumors, reflects the challenges faced in diagnosing and treating parotid neoplasms in children. PATIENTS AND METHODS: This retrospective study included all children who presented to the Children's Cancer Hospital Egypt (CCHE, 57357) with parotid masses from January 2008 to December 2020. RESULTS: Twenty-one patients were included. Malignant neoplasms were found in 12 (57.1%) of which mucoepidermoid carcinoma was the most common. Benign neoplasms were found in 6 (28.6%) all of them were pleomorphic adenoma, and non-neoplastic lesions were found in 3 (14.3%). Superficial, deep, or total parotidectomy was performed according to the involved lobes. The facial nerve was sacrificed in three cases because of frank invasion by the tumor. Neck dissection was considered in clinically positive lymph nodes and/or T3/4 masses. Complications occurred in 7 (33.3%) all were of the malignant cases. Adjuvant radiotherapy was restricted to high-risk cases (7 cases). Recurrence occurred in two cases, and one patient died of distant metastasis. Fine needle aspiration cytology (FNAC) showed 88.9% sensitivity and 100% specificity for diagnosing malignant neoplasms. The correlation of radiological and pathological staging was fair (66.74% for overall staging). CONCLUSIONS: Parotidectomy is the backbone treatment for benign and malignant pediatric parotid tumors. Neck nodal dissection should be considered after preoperative FNAC of suspicious nodes. Adjuvant radiotherapy is considered only in high-risk tumors. Preoperative FNAC of parotid masses and clinically suspicious lymph nodes is highly recommended.

Cost-effectiveness of childhood cancer treatment in Egypt: Lessons to promote high-value care in a resource-limited setting based on real-world evidence.

Background: Childhood cancer in low-and middle-income countries is a global health priority, however, the perception that treatment is unaffordable has potentially led to scarce investment in resources, contributing to inferior survival. In this study, we analysed real-world data about the cost-effectiveness of treating 8886 children with cancer at a large resource-limited paediatric oncology setting in Egypt, between 2013 and 2017, stratified by cancer type, stage/risk, and disease status. Methods: Childhood cancer costs (USD 2019) were calculated from a health-system perspective, and 5-year overall survival was used to represent clinical effectiveness. We estimated cost-effectiveness as the cost per disability-adjusted life-year (cost/DALY) averted, adjusted for utility decrement for late-effect morbidity and mortality. Findings: For all cancers combined, cost/DALY averted was $1384 (0.5 × GDP/capita), which is very cost-effective according to WHO-CHOICE thresholds. Ratio of cost/DALY averted to GDP/capita varied by cancer type/sub-type and disease severity (range: 0.1-1.6), where it was lowest for Hodgkin lymphoma, and retinoblastoma, and highest for high-risk acute leukaemia, and high-risk neuroblastoma. Treatment was cost-effective (ratio <3 × GDP/capita) for all cancer types/subtypes and risk/stage groups, except for relapsed/refractory acute leukaemia, and relapsed/progressive patients with brain tumours, hepatoblastoma, Ewing sarcoma, and neuroblastoma. Treatment cost-effectiveness was affected by the high costs and inferior survival of advanced-stage/high-risk and relapsed/progressive cancers. Interpretation: Childhood cancer treatment is cost-effective in a resource-limited setting in Egypt, except for some relapsed/progressive cancer groups. We present evidence-based recommendations and lessons to promote high-value in care delivery, with implications on practice and policy. Funding: Egypt Cancer Network; NIHR School for Primary Care Research; ALSAC.

Operative management and outcomes in children with pheochromocytoma.

OBJECTIVE: This study aimed to evaluate management and prognosis in children with pheochromocytoma who were treated at an Egyptian tertiary center. METHODS: The authors conducted an 8-year retrospective analysis for 17 patients who were presented from January 2013 to January 2021. Clinical criteria, operative details, and follow-up data were assessed. Overall (OS) and event-free survival (EFS) were estimated by the Kaplan-Meier method. An event was assigned with the occurrence of recurrence or metachronous disease, or death. RESULTS: Median age at diagnosis was 14 years (range: 6-17.5 years). Ten patients (58.8%) were males and seven (41.2%) were females. Hypertension-related symptoms were the main presentations in 15 patients (88%). None of the included children underwent genetic testing. Sixteen patients (94%) had unilateral tumors (right side: 12), whereas only one was presented with bilateral masses. The median tumor size was 7 cm (range: 4-9 cm). Metastatic workup did not reveal any metastatic lesions. All patients underwent open adrenalectomy, and clinical manifestations were completely resolved after surgery. Adjuvant therapy was not administered to any patient. There were no deaths or relapses at a median follow-up time of 40 months, whilst two children had metachronous disease after primary resection. Both were managed by adrenal-sparing surgery, and they achieved a second complete remission thereafter. Five-year OS and EFS were 100% and 88%, respectively. CONCLUSIONS: Complete surgical resection achieves excellent clinical and survival outcomes for pheochromocytoma in children. Meticulous, long-term follow-up is imperative for early detection of metachronous disease to facilitate adrenal-sparing surgery. Genetic assessment for patients and their families is essential; however, it was not available at our institution.

Management and outcome of pediatric metastatic Wilms' tumor at the National Cancer Institute, Egypt.

BACKGROUND: Wilms' tumor (WT) is the most common renal malignant tumor of childhood. Metastatic WT has a worse prognosis than localized disease. This study aims to assess the clinical outcome and different prognostic factors that influence treatment outcome of pediatric metastatic WT cases treated at National Cancer Institute (NCI), Egypt, between January 2008 and December 2015. Medical records were retrospectively reviewed for clinical, radiological and histopathological data, treatment received, and survival outcome. RESULTS: In the specified study period, 24/103 (23.3%) patients with WT were metastatic at presentation. The mean age was 5.25 ± 2.87 years (range 2.0-12.7). Abdominal swelling/mass was the commonest presentation (70.8%). Only 3 patients (12.5%) had combined lung and liver metastases while 21 patients (87.5%) had pulmonary-only metastases. All patients had favorable histology tumors with no anaplasia. Nine patients (37.5%) underwent upfront nephrectomy. Majority of patients (91.7%) had local stage III disease. Surgical complications were reported in 4 patients; 3 of them had up-front nephrectomy. Only 7/21 patients achieved rapid complete response of pulmonary nodules after 6 weeks of chemotherapy (CTH), and they had a better survival outcome. Patients were followed up till December 2017. Thirteen patients (54.1%) experienced events during the study period including 5 relapses, 6 cases with disease progression, and 2 patients died out of sepsis. The 3-year event-free and overall survival rates were 48.2% and 54.2%, respectively. CONCLUSION: Neo-adjuvant CTH followed by delayed nephrectomy seems more suitable approach in our institute. Pulmonary response to neo-adjuvant CTH appears to be a strong predictor for outcome.

Relapsed Wilms' tumor in pediatric patients: challenges in low- to middle-income countries-a single-center experience.

BACKGROUND: Wilms' tumor (WT) affects one in 10,000 children and accounts for 5% of all childhood cancers. Although the overall relapse rate for children with WT has decreased to less than 15 %, the overall survival for patients with recurrent disease remains poor at approximately 50 %. The aim of the study to evaluate the outcome of relapsed Wilms' tumor pediatric patients treated at the National Cancer Institute (NCI), Egypt, between January 2008 and December 2015. RESULTS: One hundred thirty (130) patients diagnosed with WT during the study period, thirty (23%) patients had relapsed. The median follow up period was 22.3 months (range 3.6-140 months). The Overall Survival (OS) was 30.9% while the event-free survival (EFS) was 29.8% at a 5-year follow up period. Median time from diagnosis to relapse was 14.4 months. A second complete remission was attained in 18/30 patients (60%). The outcome of the 30 patients; 11 are alive and 19 had died. Three factors in our univariate analysis were prognostically significant for survival after relapse. The first was radiotherapy given after relapse (p = 0.012). The 5-year EFS and OS for the group that received radiotherapy were 41.9% versus 16.7% and 11.1% respectively for those that did not. The second was the state of lymph nodes among patients with local stage III (p = 0.004). Lastly, when risk stratification has been applied retrospectively on our study group, it proved to be statistically significant (p = 0.029). CONCLUSION: Among relapsed pediatric WT, radiotherapy improved survival at the time of relapse and local stage III with positive lymph nodes had the worst survival among other stage III patients.

Localized Wilms' tumor in low-middle-income countries (LMIC): how can we get better?

BACKGROUND: Wilms' tumor (WT) represents about 6% of all childhood cancers. The overall survival markedly improved to exceed 90% in developed countries, yet some studies from developing counties still have poorer outcomes. The aim of this study is to assess the clinical outcome and the different prognostic factors that influence the outcome of pediatric loco-regional WT cases treated at National Cancer Institute (NCI), Cairo University, Egypt. This is a retrospective study which included pediatric loco-regional WT patients presented between January 2008 and December 2017. Patients were followed up till June 2019. RESULTS: Ninety-two eligible patients were included. Median age was 3 years (range 1 month-9 years). Abdominal mass was the commonest presentation (72.8%). The 5-year EFS and OS of the whole group was 83.7% and 94.6% retrospectively. Despite having a similar EFS (84.8 vs. 82.6%), stage III patients had a significantly lower OS than those in stages I and II (89.1% vs. 100%, p value 0.024). Twelve patients had unfavorable histology and had a significantly lower EFS and OS than the patients with favorable histology (50 and 83.3% vs. 88.8 and 96.3%, p value < 0.001 and 0.043, respectively). CONCLUSION: Loco-regional Wilms' tumor cases treated in Egypt had OS nearly the same as in developed countries, but had a lower EFS than expected mainly stages I and II. The stage and histological type are the main factors influencing the survival, and further studies are needed to investigate nuclear unrest grades and proper management of such cases.

Treatment and Outcome in 65 Children with Optic Pathway Gliomas.

INTRODUCTION: Optic pathway gliomas (OPGs) are rare neoplasms in children with an unpredictable clinical course. There is significant controversy regarding the optimal management and outcome of these patients. METHODS: Charts of all patients with OPG diagnosed and treated at Children's Cancer Hospital Egypt between July 2007 and July 2014 were retrospectively reviewed. We evaluated the roles of surgical, ophthalmologic, endocrinologic, neurologic, and treatment aspects of care. RESULTS: Sixty-five patients were included in this study, with a mean age of 5.3 years. OPGs were chiasmatic (n = 25), optic nerve (n = 18), hypothalamic (n = 7), and chiasmatic/hypothalamic (n = 7). Extensive involvement of the optic pathway was seen in an additional 8 patients. Twenty cases had neurofibromatosis type 1. Four cases underwent surgical debulking, and 28 were biopsied (16 open, 11 stereotactic, and 1 endoscopic). Nine of the 18 optic nerve tumors were managed by total excision. Twenty-four patients did not undergo any surgical intervention. Forty-five patients received chemotherapy. Histopathology revealed pilocytic (n = 20), pilomyxoid (n = 15), fibrillary astrocytoma (n = 4), and grade I papillary-glioneuronal tumor (n = 1). Nonrepresentative sample (n = 1). The 4-year overall survival rate was 86.3% with mean follow-up period of 32.2 months. CONCLUSION: The initial role of surgery in newly developed OPG is biopsy for tissue diagnosis and relief of the hydrocephalus, if present, followed by chemotherapy. Chemotherapy decreases or stabilizes the tumor size in most cases, leading to preservation of both visual and endocrinal functions. The most significant prognostic factor confirmed in this study was the age of the patient.